原发性进行性失语一例临床分析

Primary progressive aphasia:a case analysis

目的 原发性进行性失语 (PPA)是一种少见的中枢神经系统变性疾病 ,国内罕见报道。现报道 1例 ,以提高临床医生对该病的认识。方法 采用韦氏记忆量表、认知能力筛选检查、积木测验、数字广度测验和社会功能问卷等全套神经心理学量表方法 ,检查和描述了PPA的临床和神经心理学特征 ;并进行了MRI和PET影像学检查。结果 病人除有单纯性命名性失语外 ,不伴有其他类型的失语与智能损害及神经系统体征 ;MRI和PET检查均发现左颞叶明显萎缩。结论 PPA以缓慢进行性失语而不伴有认知功能障碍和神经系统体征为特点 ,优势半球局灶性额、颞叶病变有诊断意义。

Objective Primary progressive aphasia (PPA) is a rare neurodegenerative disease barely reported in China. Methods Complete neuropsychological testing on memory, cognitive function and aphasia was carried out. The brain function and structure was also examined with MRI and PET imaging analysis. Results The characteristics of clinical manifestation, neuropsychological testing and brain imaging analysis were reported. Except anomic aphasia, no other abnormal neuropsychological testing as well as sign of the nervous system was found. However, a significant atrophy and decrease in glucose metabolism of the left temporal lobe was observed.Conclusion PPA is clinically characterized by progressive anomic aphasia without impairment of the cognitive function and other abnormal sign of central nervous system. Atrophy of the frontal and temporal lobe in the dominant hemisphere provides strong support for the diagnosis.